BackgroundThe objective of this study was to evaluate the dental and oral manifestations in patients with celiac disease.Material and MethodsThe sample consisted of 40 patients with the disease and 40 without the disease matched by age in southern Brazil. The CD group included patients previously diagnosed by positive anti-endomysial (IgA) examination and confirmed by small intestine biopsy. The presence of dental enamel defects and dental caries was evaluated by a calibrated researcher according to AINE’s and WHO’s criteria, respectively. The history of recurrent aphthous ulcers and dry mouth was obtained through reporting. For the evaluation of the salivary flow, the saliva samples were obtained through the non-stimulated and stimulated saliva collection method.Results There was a significant association between CD and dental enamel defects (OR=2.38, P=0.045) and dry mouth (OR=9.15, P=0.002). No difference was found for the report of recurrent aphthous ulcers and caries experience between the two groups. Patients with CD had normal pattern of unstimulated and stimulated saliva flow rates (0.67 ± 0.38 ml / min and 1.14 ± 0.47 ml / min, respectively). A higher occurrence of dental enamel defects was observed in patients with classic CD (P=0.054). Of the 1,962 permanent teeth, 59 presented dental enamel defects, 71.8% of which were in patients with CD (P=0.001), predominantly in molars (P=0.009).ConclusionsCD increased the likelihood of dental enamel defects and dry mouth sensation. The oral examination can be an important auxiliary tool for the identification of cases of the disease. Key words:Celiac disease, oral manifestations, dental enamel hypoplasia.

Down syndrome is the most common human chromosomal disorder. Among clinical findings, one constant concern is the high prevalence of gastrointestinal system alterations. The aim of this study was to determine the prevalence of gastrointestinal disorders at a Down syndrome outpatient clinic during a 10-year follow-up period. Data from medical files were retrospectively reviewed from 1,207 patients. Gastrointestinal changes occurred in 612 (50.7%). The most prevalent disorder was chronic intestinal constipation. Intestinal parasite occurred in 22% (mainly giardiasis), gastroesophageal reflux disease in 14%, digestive tract malformations occurred in 5%: 13 cases of duodenal atresia, 8 of imperforate anus, 4 annular pancreases, 2 congenital megacolon, 2 esophageal atresias, 2 esophageal compression by anomalous subclavian and 1 case of duodenal membrane. We had 38/1,207 (3.1%) patients with difficulty in sucking and only three with dysphagia that resolved before the second year of life. Peptic ulcer disease, celiac disease, and biliary lithiasis were less prevalent with 3% each. Awareness of the high prevalence of gastrointestinal disorders promotes outstanding clinical follow-up as well as adequate development and greater quality of life for patients with Down syndrome and their families. K E Y W O R D S congenital malformation, Down syndrome, gastrointestinal diseases

Introduction Dysphagia is a common symptom in children with cerebral palsy, either in oral or pharyngeal phases. Children who face such difficulties tend to show health problems such as food aspiration, malnutrition and respiratory infections. Videofluoroscopic swallowing study is the most recommended for these cases, as it reveals the real situation during swallowing. Objective The study aimed to verify the occurrence of aspiration pneumonia in children with cerebral palsy after videofluoroscopy. Methods The population for this prospective cross-sectional study involved 103 children with cerebral palsy, referred for videofluoroscopic who had returned for medical examination after a week to search for signs and symptoms of pneumonia. Results The study involved 46 girls (44.66%) and 57 boys (55.34%), aged between 0 and 14 years of age. Of the total, 84 (81.5%) had dysphagia, of which 24 (23.3%) were severe, 8 (7.7%) were moderate and 52 (50.4%) were mild dysphagia. None of the children presented aspiration pneumonia or infectious complications during the course of videofluoroscopy or after the procedure. Conclusion In the population studied, the authors found no cases of aspiration pneumonia, even with tracheal aspiration present in 32 (31.07%) cases.

Summary Introduction:?The literature reports that when it comes of instrumental assessment of swallowing in children, undoubtedly, video fluoroscopy of swallow offers great advantages over the endoscopic study. Objective:?Check the risk of aspiration pneumonia after the study of swallowing by video fluoroscopy, in children with dysphagia. Method:?In a study of prospective cutting, participated 16 children aged between 6 months and 10 years, with an average of 5,2 years, referred for study of swallowing by video fluoroscopy. Were tested 4 consistencies, pudding, nectar, honey and liquid. The presences of signs and/or respiratory symptoms were evaluated pre and post study of deglutition by video fluoroscopy, through history and clinical exam. When necessary was asked chest x-ray. Results:?Of 16 children, 5 didn't presented dysphagia. In 11 children the exam showed 4 with mild dysphagia, 2 moderate and 5 severe, as classification of OTT (1996) - Classification of severity of dysphagia to the video fluoroscopy. Of the 7 children who aspirated during the exam, only 1 presented respiratory symptoms after the deglutition study, but without signal of pneumonia to the physical examination. Conclusion:?In the studied population there were no occurrences of aspiration pneumonia after the study of deglutition was performed by video fluoroscopy, despite the occurrence of aspiration during the exam in about 50% of cases.

OBJETIVO: A reconstituição biliar no transplante hepático intervivos é associada à elevada taxa de complicações. O objetivo do presente estudo é apresentar a nossa experiência com as complicações biliares pós-transplante hepático intervivos e o seu tratamento. MÉTODO: De um total de 300 transplantes hepáticos, 51 (17%) foram com doadores vivos. Todos receptores tinham o grupo sangüíneo ABO idêntico aos dos doadores. Os prontuários eletrônicos dos receptores foram avaliados para determinar a presença e o tipo de anomalia da via biliar, o tipo de reconstituição da via biliar, presença de complicações vasculares e biliares e o método e o resultado do tratamento das complicações. RESULTADOS: A via biliar era dupla em sete enxertos (16,7%) e tripla em dois (4,8%) enxertos do lobo hepático direito. Nos demais, ela era única. O tipo de reconstituição mais comum foi a hepaticohepaticostomia única ou dupla (38 transplantes; 75%). Complicações biliares ocorreram em 21 pacientes (41,2%) e incluíram fístula biliar em 11 (21,6%), estenose biliar em seis (11,8%) e fístula com estenose em quatro (7,8%). O local da fístula foi na anastomose biliar em 11 pacientes (21,6%) e na superfície cruenta do fígado em quatro (7,8%). O tratamento consistiu de inserção de prótese biliar em oito, papilotomia em um, retransplante em dois que tinham trombose da artéria hepática e sutura do ducto em um. A fístula fechou com o tratamento conservador em três pacientes. A maioria dos pacientes com estenose biliar foi tratada com dilatação seguida da colocação de prótese biliar. CONCLUSÕES: As complicações biliares são freqüentes após o transplante hepático intervivos e são associadas à elevada taxa de morbidade e mortalidade.

BACKGROUND: Molar incisor hypomineralization (MIH) is a developmental enamel defect with multifactorial etiology. Although the relationship between celiac disease (CD) and developmental enamel defect was demonstrated, the association between CD and MIH is uncertain. OBJECTIVE: The objective of this study was to analyze the occurrence of MIH in CD patients. METHODS: Forty CD patients and a control group with 40 healthy individuals were selected. A calibrated examiner (k≥0.889) according to the European Academy of Pediatric Dentistry criteria performed the diagnosis of MIH. Data were analyzed by descriptive statistics and Fischer’s exact test (α=0.05). RESULTS: Of the 80 participants, ten presented MIH with eight individuals with CD. Celiac patients presented 4.75 times the chance of occurrence of MIH than the control group (95% CI: 2.22-10.18; P=0.044). In all the evaluated teeth (n=978), 22 had MIH: 20 teeth in individuals with CD and two in those without the disease. All CD participants with MIH presented the classic form of the disease. CD participants showed 17 teeth (85.0%) with demarcated opacities, two (10.0%) post-eruptive collapses and one (5.0%) atypical restoration. The control group presented only demarcated opacities. CONCLUSION: CD increased the chance of MIH and associated with its clinical manifestations can assist in the diagnosis of CD.

As transformações biopsicossociais da adolescência colocam os adolescentes transplantados de fígado em maior risco de não aderência ao tratamento e piora da evolução, quando comparados a outros grupos etários. Objetivo: Verificar a influência de fatores psicológicos do paciente e sua família na aderência ao imunossupressor após o transplante hepático. Métodos: Sete instrumentos psicológicos foram aplicados a 30 pacientes transplantados hepáticos adolescentes e adultos jovens (12 a 30 anos): escala de autoestima, escala de resiliência, escala de qualidade da interação familiar, escala de satisfação com a vida, escala de orientação de vida, escala de autoeficácia percebida, escala de exigência e responsividade e escala de qualidade de interação familiar. Resultados: Na avaliação por regressão logística multivariada, as variáveis orientação de vida e resiliência foram as principais características que interferiram na rejeição clínica (p = 0,03). As variáveis do pai que atuam na boa aderência (p = 0,03) foram: comunicação negativa, responsividade, clima conjugal positivo, punição física e comunicação positiva, enquanto que envolvimento, sentimento dos filhos e comunicação positiva aumentaram a rejeição clínica (p<0,01). Para a mãe, responsividade, comunicação positiva e punição física tended to adhesion (p=0,06), enquanto que a punição física e a comunicação negativa aumentaram a possibilidade de rejeição clínica (p = 0,02). Conclusões: O perfil psicológico do paciente e da família interfere na adesão ao tratamento imunossupressor e na rejeição pós-transplante hepático e poderia ser utilizado para triagem de pacientes com risco de má aderência ao tratamento imunossupressor e consequente rejeição ao enxerto.

The Hutchinson-Gilford syndrome or progeria is a rare autosomal dominant syndrome characterized by premature aging and involvement of internal systems, such as the circulatory and locomotor. The diagnosis is essentially clinical and the manifestations become more evident from the first year of life. Long term outcome data from Progeria Research Foundation clinical trials have demonstrated an increase in survival in recent years. Even though new trials are ongoing, the recognition of this syndrome is essential to prevent cardiovascular and cerebrovascular complications. A patient, initially asymptomatic, who developed characteristic signs of the syndrome at the age of 6 months is reported. She was referred for evaluation only when she was two years and eleven months old. The diagnosis of Hutchinson-Gilford syndrome was suspected owing to clinical characteristics. The diagnosis was confirmed by genetic testing. A mutation c.1824C> T in exon 11 of the LMNA gene was detected. She was registered in the Progeria Research Foundation and was invited to participate in the weighing and supplementation program. She was included in the lonafarnib protocol study. This medication is a farnesyl transferase inhibitor that prevents the production of progerina and slows cardiovascular and neurological complications of the syndrome. This case highlights the importance of diagnosing progeria patients because they may be referred to the Progeria Research Foundation, which offers genetic screening and inclusion in clinical and therapeutic follow-up protocols without any costs. Progeria trials and research may also contribute to new drug developments related to prevention of aging and atherosclerosis in the near future.